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Renal cell carcinoma with massive osseous metaplasia and bone marrow elements.

Focal calcifications are frequently seen in renal masses and may be present in renal cell carcinomas. Metaplastic bone formation, on the other hand, is a rare event. We report a unique case of a large calcified renal cell carcinoma with massive osseous metaplasia and bone marrow elements. The clinical and pathologic differential diagnosis for this tumor is discussed along with a review of the literature on this unusual phenomenon.

Biomarkers, Tumor↗

The Upper Paleolithic triple burial of Dolní Vestonice: pathology and funerary behavior.

This work focuses on paleopathological analysis of one of the skeletons from the Gravettian triple burial of Dolní Vestonice (Moravia) and addresses issues of Upper Paleolithic funerary behavior. The burial includes the well-preserved skeletons of three young individuals. The skeleton in the middle (DV 15) is pathological and very problematic to sex; the other two (DV 13 and DV 14) are males and lie in an unusual position. The young age, the possibility of a simultaneous interment, and the position of the three specimens have given rise to speculations about the symbolic significance of this spectacular and intriguing funerary pattern. The pathological condition of the skeleton in the middle further emphasizes its peculiarity. Main pathological changes of the DV 15 skeleton include: asymmetric shortening of the right femur and of left forearm bones, bowing of the right femur, right humerus, and left radius, elongation of fibulae, dysplasias of the vertebral column, and very marked enamel hypoplasias. Scrutiny of the medical literature suggests that the most likely etiology is chondrodysplasia calcificans punctata (CCP) complicated by trauma and early fractures of the upper limbs. CCP is a rare inherited disorder characterized by stippled ossification of the epiphyses. The cartilaginous stippling is a transient phenomenon that disappears during infancy, leaving permanent deformities on affected bones. Among the different forms of CCP, the X-linked dominant form is that resulting in asymmetric shortening and is lethal during early infancy in males. Thus, survival of DV 15 until young adult age would require the specimen to be a female. Clinical findings often associated with the disease (erythemas, ichthyosis, alopecia, cataracts, and joint contractures, among others) would emphasize the singular aspect of this individual, pointing to a condition that should be carefully taken into account when speculating on the significance of that peculiar burial.

Adult↗

The painful accessory navicular.

The accessory navicular is usually considered a normal anatomic and roentgenographic variant. The term may refer to two distinct patterns. First, a sesamoid bone may be present within the posterior tibial tendon (Type 1); this is anatomically separate from the navicular. Second, an accessory ossification center may be medial to the navicular (Type 2). During postnatal development this is within a cartilaginous mass that is continuous with the cartilage of the navicular. At skeletal maturity the accessory center usually fuses with the navicular to form a curvilinear bone. The Type 2 pattern may be associated with a painful foot, particularly in the athletic adolescent, and should not be arbitrarily dismissed as a roentgenologic variant in the symptomatic patient. The clinical, radiologic, pathologic, and surgical findings in ten cases are reviewed. Roentgenographically the ossicle is triangular or heart-shaped. 99mTc MDP imaging may be of value when the significance of the ossicle is uncertain. Even when the roentgenographic variant is bilateral, increased radionuclide activity occurs only on the symptomatic side. Histologic examination of surgically excised specimens reveals inflammatory chondro-osseous changes in the navicular-accessory navicular synchondrosis compatible with chronic trauma and stress fracture. Nonsurgical treatment with orthotics or cast immobilization produces variable results and resection of the accessory navicular may be the treatment of choice.

Adolescent↗

Vitamin C deficiency causes hematological and skeletal abnormalities during fetal development in swine.

The influence of maternal vitamin C deficiency on fetal development was studied in swine with a hereditary lack of ability to synthesize ascorbic acid (OD pigs). Thirteen pregnant sows homozygous (od/od) for the defect were depleted of ascorbic acid for 24 to 38 d at various stages of gestation. Six normal (OD/OD) sows were used as controls. Only a few experimental sows showed clinical symptoms of vitamin C deficiency. Nevertheless, severe pathological changes were seen in the uterus and fetuses. Characteristic findings were hemorrhages and hematomas in both fetal and maternal placenta, and general edema and subcutaneous hemorrhages in the fetuses. Similarities were noted to the abruptio placentae syndrome in women. Depletion of vitamin C resulted in a pronounced decline in ascorbic acid concentration in most maternal and fetal organs as well as in plasma and embryonic fluids. No morphological malformations were found in the fetuses, but the ossification of the skeleton was severely deranged. Macroscopically the lesions comprised swelling of the costochondral junction and separation of the epiphysial cartilage from the spongiosa in ribs and limb bones. Another characteristic finding was loosening of the periost from the cortex, often resulting in subperiosteal bleedings. Microscopically normal osteoblasts were few and the formation of osteoid defective.

Animals↗

Experimental chronic compression on the spinal cord of the rabbit by ectopic bone formation in the ligamentum flavum with bone morphogenetic protein.

This study was conducted to induce chronic spinal cord compression myelopathy in rabbits. The L5 lumbar lamina was cut partially in 70 rabbits, and bone morphogenetic protein (BMP) was implanted on the ligamentum flavum in 35 of them. In the BMP group, new bone formed on the dorsal side of the spinal canal and flattened the spinal cord in an anteroposterior direction. No pathological changes were detected in the intramedullary tissues by light microscopic examination. In rabbits it is possible to induce compression of the cord by using BMP, although sufficient cord compression to induce myelopathy was not achieved.

Animals↗

[Vascularization of the laryngeal cartilages; true clinical importance (author's transl)].

The authors specify the role of the ossification process of the larynx cartilages. Only the epiglottis may be considered as a cartilage. The use of microangiography made it possible to describe some peculiar features of the microvascular pattern. The vascular patterns of the thyroid, cricoid and arithenoids cartilages are similar to one another; the cartilage and its two perichondriums must be considered as a complete whole. The epiglottis shows a peculiar vascular pattern. The authors insist upon the important role of the vascular pattern in pathologic studies.

Angiography↗

Expression and distribution of transforming growth factor-beta isoforms and their signaling receptors in growing human bone.

Transforming growth factors type beta (TGF-beta1, -beta2, and -beta3) are potent stimulators of bone formation and have been shown to regulate chondrocyte, osteoblast, and osteoclast formation and function. However, the distribution of the different isoforms and their signaling receptors in human bone in vivo has not previously been reported. Using samples of normal (neonatal rib) and pathological (osteophytic) developing human bone, we have investigated the expression of the different TGF-beta isoforms and their signaling receptors (TGF-betaRI and RII) at the messenger ribonucleic acid (mRNA) and protein levels by in situ hybridization and immunolocalization to establish the sites of TGF-beta production and their possible sites of action during human bone development in vivo. All three TGF-beta isoforms and the receptors were detected at sites of endochondral and intramembranous ossification. At sites of endochondral ossification, TGF-beta2 was detected in all zones of the cartilage, with the highest expression seen in the hypertrophic and mineralizing zones. TGF-beta3 was detected in proliferative and hypertrophic zone chondrocytes, while TGF-beta1 expression was restricted to the proliferative and upper hypertrophic zones. TGF-betaRI and RII exhibited similar distributions with maximum expression in the hypertrophic and mineralizing zones in the neonatal rib but in the resting/proliferative zone in the developing osteophyte. At sites of intramembranous ossification TGF-beta3 was the most widely distributed isoform and showed both matrix- and cell-associated staining. TGF-beta2 and -beta1 were expressed almost exclusively at sites of mineralization. These observations demonstrate that the different TGF-beta isoforms and their receptors exhibit distinct but overlapping patterns of expression, and support the hypothesis that they are involved in the regulation of endochondral and intramembranous ossification during human bone development in vivo.

Activin Receptors, Type I↗

Primary osteosarcoma of the kidney.

Osteosarcoma arising in the kidney is a bona fide entity. A case is reported and described in clinical and pathological detail. Characteristic changes include a palpable stony hard renal mass associated with radiographic demonstration of sunburst calcification in the kidney. The case presented is unique in that virtually the entire specimen was composed of bone.

Humans↗

Posttraumatic contracture of the elbow: current management issues.

Posttraumatic elbow stiffness can impose severe functional limitations on the performance of activities of daily living. Prevention is key to avoiding a motion-limiting condition. Fractures should be anatomically reduced and stabilized with active and active-assisted range of motion exercises instituted as early as possible to minimize the development of stiffness. Established contractures should be treated initially with physical therapy and static-progressive splinting. Patients who have failed a minimum of six months of nonsurgical management and who are motivated to comply with postoperative rehabilitation are candidates for surgical release. There are several effective surgical approaches and techniques available. The choice of surgical approach and technique is dictated by the location of the pathology, condition of the skin, and degree of arthritic changes. A major challenge to care is the management of the young patient with posttraumatic elbow contracture and advanced degenerative changes for which there is currently no reliable long-term surgical treatment.

Arthroscopy↗

[Problems and results of skull x-ray following vacuum extraction (author's transl)].

The vacuum extraction supplies the forceps nearly complete on the IInd Department of obstetrics and gynecology Vienna in the last years. Skull X-ray of 104 newborns following vacuum extraction and of 101 spontaneous delivered newborns are examined on the incidence of fractures, fracture-fissures, elevations and widening of skull sutures. Early pathological findings are rigid criteria for the possible danger due to a method of operative delivery but we have no typical late damage after vacuum extraction or forceps delivery, we don't find in spontaneous born children too.

Birth Injuries↗

Focal osteolysis in total hip replacement: CT findings.

OBJECTIVE: To describe the CT findings of focal osteolysis following total hip replacement (THR). DESIGN AND PATIENTS: CT imaging features of 30 THRs with focal osteolysis visualized on follow-up radiographs and undertaken revision surgery were reviewed. On CT scans, the shape and anatomic location of osteolytic lesions was recorded, as well as their size and number. The presence of cortical disruption or expansion, liner wear, metallosis, and heterotopic ossification was also noted. In each case, surgical and histologic findings were correlated with imaging features. RESULTS: Focal osteolysis was common in the superior part of acetabular and femoral components. CT features of focal osteolysis were multiple, expansile, oval, or round radiolucencies, which were conglomerated into multilobular shape. The cortex adjacent to the osteolytic lesions revealed irregular thinning and discontinuity (29/30, 97%) accompanied by a few tiny fragments. Liner wear was common (27/30, 90%), and metallosis was frequent (8/30, 26.7%). Pathologic results were foreign body reaction in 20 patients, chronic inflammation in eight, and fibrosis in two. CONCLUSION: CT appearances of focal osteolysis following THR are multilobulated lucent areas with expansile periosteal reaction and cortical abnormalities, mimicking infection or tumor. We consider that CT is useful for the prediction and assessment of the nature and extent of focal osteolysis.

Acetabulum↗

Fibro-osseous lesions of the external auditory canal.

OBJECTIVES: To differentiate a novel type of benign circumscribed bone lesion of the external auditory canal from those described previously, such as exostoses and osteomas. STUDY DESIGN: Information was obtained from computed tomography (CT) images, surgical findings, and pathologic study. METHODS: Five patients (26 to 82 years old) who presented a hard, round, unilateral, skin-covered mass occluding the external auditory canal to varying degrees were studied. A CT study carried out before resection of the lesions by curettage disclosed the absence of a bony connection to the underlying structures. All the tissue specimens underwent pathologic study. RESULTS: CT and surgical findings demonstrated the absence of a connective pedicle. The pathologic findings showed lesions consisting of an osteoma-like bone formation with sparse osteoblastic areas; mature lamellar bone was observed in three cases, bone marrow containing adipose tissue and hematopoietic remnants in two, and a dense, collagenous stroma in another. They all showed irregular trabeculae, bordered by osteoid osteoblasts. In no case was there evidence of a relationship to the cartilaginous tissue or to the bony structures of the external auditory canal. CONCLUSIONS: The data obtained from the clinical, CT, surgical, and pathologic findings suggest the existence of a lesion unlike those previously known, possibly related to ossifying reactions in other parts of the organism.

Adult↗

Adenocarcinoma of the urachus showing extensive calcification and stromal osseous metaplasia.

We report a case of adenocarcinoma of the urachus showing extensive calcification and stromal osseous metaplasia, arising in the bladder dome of a 51-years-old man. The patient underwent partial cystectomy, including in block excision of the entire urachus and umbilicus, and remains well, free from symptoms after a 22-month follow-up period. Main pathologic and clinical characteristics of urachal calcified tumors are reviewed. To our knowledge, this is the first report in the English literature on urachal carcinoma showing both extensive scattered calcification and massive stromal osseous metaplasia.

Adenocarcinoma↗

Pseudoachondroplastic dysplasia in miniature poodles: clinical, radiologic, and pathologic features.

The clinical, radiologic, and pathologic features of pseudoachondroplastic dysplasia were studied in 13 affected Miniature Poodles. The disease became evident when pups were about 3 weeks old. Normal-appearing and affected pups were in the same litter, and both sexes were affected. Abnormal locomotion with abducted hindlimbs and flattened rib cages were accompanied by short, bent limbs and enlarged joints. Radiographically, areas of stippling and patchy densities were evident in the epiphyses. Histologically, these densities were identified as areas of bone associated with retarded ossification. After growth is completed, the epiphyses, ossify, but the bones are shortened and deformed. General health appears normal, except for restricted locomotion due to secondary arthritis and difficult breathing in dogs with a deformed thorax.

Achondroplasia↗

Adolescent idiopathic scoliosis: an update.

Adolescent idiopathic scoliosis is defined as a lateral spinal curvature of greater than 10 degrees, for which no pathologic cause can be determined. The initial assessment of adolescents with scoliosis focuses on identification of any treatable underlying pathology. Adolescents with scoliosis typically are asymptomatic and have normal neurologic and physical examinations, with the exception of curvature of the spine. Treatment strategies are determined by the risk of progression. This risk depends on the extent of the curvature and anticipated future spinal growth. The extent of the curvature may be estimated by use of a scoliometer and verified by calculation of the Cobb angle on radiographic evaluation. Skeletal maturity may be estimated by several methods, including radiologic estimates of ossification by bone atlas or Risser sign. Treatment strategies include bracing and surgery.

Adolescent↗

Nonunited ossification center of the presphenoid bone: pseudomeningioma.

Three patients are described; in two of them initial plain skull films at an outside institution were interpreted as demonstrating an anterior clinoid meningioma. Evaluation of these films along with further study of the skull base with complex motion tomography demonstrated a separate and well corticated bony structure posterior and superior to each anterior clinoid in all three cases. After review of the developmental anatomy of this region, it was concluded that these bone centers represent failure of the posterior accessory centers of the presphenoid to fuse with the orbitosphenoid. The features of these nonunited centers appear to be characteristic and should allow distinction of this developmental anomaly from a pathologic process.

Adult↗

Symptomatic ossification of the ligamentum flavum: a clinical series from the French Antilles.

STUDY DESIGN: A series of 14 patients from the French Antilles treated for ossification of the ligamentum flavum (OLF). OBJECTIVES: To describe the clinical and radiologic aspects, as well as disease course in a group of Caribbean patients. Also describe the use of sagittal computerized tomography (CT) reconstructions to distinguish OLF from calcification of the ligamenta flava. SUMMARY OF BACKGROUND DATA: OLF is a rare disease described almost exclusively in Japanese patients. Only rarely are patients of African descent affected. No series of OLF in African American or African Caribbean subjects has previously been published. METHODS: A retrospective study of 14 consecutive patients, including 7 men and 7 women (mean age, 66.8 years), was conducted from 1996 to 2003. Diagnosis in each case was established using CT. Magnetic resonance imaging was also performed in every case. For the 11 patients treated surgically, pathology studies were performed. RESULTS: Walking difficulties were the most common presenting complaint. A picture of spastic paraparesis associated with sphincter dysfunction was the most common finding on initial examination. In each case, CT provided sufficient information to establish a diagnosis of OLF, while magnetic resonance imaging was helpful for showing spinal cord involvement. In most of the patients, OLF was located in the lower thoracic spine. Surgical decompression through a posterior approach resulted in regression of symptoms in all 11 patients treated surgically. CONCLUSIONS: This study is the first reported series of OLF in a group of Caribbean patients. The disease appears to be underreported in the African Caribbean population. OLF can lead to debilitating thoracic myelopathy. Surgery is frequently indicated and achieves favorable results.

Adult↗

Calcification of the aortic valve: its progression and grading.

Three hundred and seventy four aortic valves which had been surgically removed over the past five years were studied by routine histology. Most patients were male and over the age of 60 years. There were 3.7% bicuspid valves, 16% valves with evidence of past rheumatic fever and 2.1% with endocarditis. A range of pathological lesions was seen including calcification, chondroid and osseous metaplasia, neovascularization, inflammation and cholesterol deposition. A common lesion was a progressive dystrophic calcification of the valve cusps. This was studied and graded in relationship to the concomitant structural damage. There has been no previously published grading system for this type of pathological change in the aortic valve. Our criteria for the four grades of aortic valve lesion are described. Most patients were found to have lesions of Grades 3 and 4.

Adult↗